Marta Carlesimo

Treatment of cutaneous and pulmonary sarcoidosis with thalidomide

Many therapeutic agents have been proposed for treatment of steroid-resistant sarcoidosis. Because administration of low doses of thalidomide has been successful in treating other inflammatory diseases, it was used in a patient with systemic sarcoidosis who was unresponsive to corticosteroids and in a patient with pulmonary sarcoidosis, in whom Kaposis sarcoma developed after a course of corticosteroid therapy. Thalidomide, 200 mg/day for 2 weeks followed by 100 mg/day for 11 weeks, was given. This treatment was effective in both patients. No adverse reactions were observed. Thalidomide, 100 mg on alternate days, is still being administered. No relapse has occurred. Thalidomide, particularly because of its inhibition of the macrophage function, may be a useful alternative therapy in steroid-resistant cases. In addition, the correlation between the angiotensin-converting enzyme level and the clinical improvement observed in our patients suggests a direct parallel between angiotensin-converting enzyme and the activity of the granulomatous process.

Comparitive Effectiveness and Finasteride Vs Serenoa Repens in Male Androgenetic Alopecia: A Two-Year Study

The objective of this open label study is to determine the effectiveness of Serenoa repens in treating male androgenetic alopecia (AGA), by comparing its results with finasteride. For this purpose, we enrolled 100 male patients with clinically diagnosed mild to moderate AGA. One group received Serenoa repens 320 mg every day for 24 months, while the other received finasteride 1 mg every day for the same period. In order to assess the efficacy of the treatments, a score index based on the comparison of the global photos taken at the beginning (T0) and at the end (T24) of the treatment, was used. The results showed that only 38% of patients treated with Serenoa repens had an increase in hair growth, while 68% of those treated with finasteride noted an improvement. Moreover finasteride was more effective for more than half of the patients (33 of 50, i.e. 66%), with level II and III alopecia. We can summarize our results by observing that Serenoa repens could lead to an improvement of androgenetic alopecia, while finasteride confirmed its efficacy. We also clinically observed, that finasteride acts in both the front area and the vertex, while Serenoa repens prevalently in the vertex. Obviously other studies will be necessary to clarify the mechanisms that cause the different responses of these two treatments.

Monilethrix treated with minoxidil.

In literature many different therapies are proposed to treat Monilethrix, but a definitive therapy still doe not exist. We decided to treat four patients affected by Monilethrix, with topical minoxidil 2%, 1 ml night and day for 1 year. Minoxidil led to a an increase of normal hair shaft without any side effects in all the patients. Therefore topical minoxidil 2% could be considered a good therapy to treat Monilethrix.

Erythema annulare centrifugum: report of a case with neonatal onset

Dariers erythema annulare centrifugum (EAC) is often associated with infectious, autoimmune or neoplastic disease, nevertheless, most cases of EAC remain unexplained.

Skin diseases in immigrants seen as out-patients in the Institute of Dermatology of the University of Rome 'La Sapienza' from 1989 to 1994

In the Institute of Dermatology of the University of Rome ‘La Sapienza’ (IDURLS) from 1989 to 1994, 2198 immigrants from countries outside the European Community were seen as out-patients. Their personal and pathological data were collected by a particular software ‘Questio’, set up together with colleagues of the Interdepartimental Center for Scientific Calculous (ICSC) of the University of Rome ‘La Sapienza’. The number of patients has been increasing from 326 in 1989 to 436 in 1994. Most of the people were coming from Africa, but from 1989 to 1994 the number of patients from the regions of ex-Yugoslavia has been increasing. About one third of the immigrants were unemployed, whereas 40% of them had a job (worker or employee), and the remaining 20% was composed by students and housewives. Most immigrants (1474 patients) were 20–39-year-old. Their data were compared with those of a control group composed by 2100 Italian people of the same age and sex, seen as out-patients in IDURLS in the same period. The most frequent skin disorders observed in immigrants were skin infectious or parasitic diseases, above all scabies. Excluding people with scabies, both immigrants and Italian patients shared the same skin diseases typical of young adult people: many fungal infections, many contact dermatitis and very few skin malignant tumors.

Videodermoscopy Scalp Psoriasis Severity Index (VSCAPSI): A useful tool for evaluation of scalp psoriasis

The standard methods used to diagnose scalp psoriasis vary in sensitivity, reproducibility, and invasiveness. Videodermoscopy can be used to explore microcirculatory modifications in skin diseases. Psoriasis presents three pathognomonic vascular patterns: red dots, hairpin vessels and red globular rings. Our aim was to create a videodermoscopy scalp psoriasis severity index (VSCAPSI) for evaluation of scalp psoriasis, especially mild and moderate forms that often are not clinically appreciable. VSCAPSI takes into account the area of the scalp affected by psoriasis, the presence and morphology of vascular patterns, the erythema and desquamation. Videodermoscopy images obtained between November 2009 to June 2010 from 900 participants with various scalp and hair disorders were reviewed for distinguishing features. During the 2010 Italian congress on psoriasis, in order to assess the reproducibility and efficacy of the VSCAPSI, 146 dermatologists were asked to evaluate 16 videodermoscopy images of scalp psoriasis using the VSCAPSI. Of the 900 patients, 85 new cases of scalp psoriasis were diagnosed. The other 815 patients were found to be suffering from different scalp and hair diseases. Of 146 dermatologists, 28 did not recognize erythema, 15 desquamation and 7 the vascular patterns. The VSCAPSI provides important evidence for early diagnosis, differential diagnosis, for follow-up and screening.

Conditions simulating androgenetic alopecia.

Androgenetic alopecia is a common form of hair loss, characterized by a progressive hair follicular miniaturization, caused by androgen hormones on a genetically susceptible hair follicle, in androgenic‐dependent areas. Characteristic phenotype of androgenetic alopecia is also observed in many other hair disorders. These disorders are androgenetic‐like diseases that cause many differential diagnosis or therapeutic error problems. The objective of this review was to systematically analyse the greatest number of conditions that mimic the AGA pattern and explain their disease pathogenesis.

Griscelli syndrome type 2: long-term follow-up after unrelated donor bone marrow transplantation.

Griscelli syndrome (GS) is a rare autosomal recessive disease characterized by silvery hair (‘partial albinism’). Three forms exist; GS type 2 (GS2), the most common one, is characterized by severe primary immunodeficiency with acute episodes of hemophagocytic lymphohistiocytosis (HLH) which may be fatal in the absence of hematopoietic stem cell transplantation. A 5-year-old boy with HLH was referred to us because of silvery-gray hair present since birth. Abnormal pigment clumps were observed in the medulla of hair shafts on light microscopy. Electron microscopy of a skin biopsy revealed melanosomes in melanocytes, but not in keratinocytes. Leukocytes were devoid of intracytoplasmic granules on blood smear. Neurological signs were absent. Genotyping revealed a homozygous haplotype for polymorphic markers linked to the RAB27A locus, but no RAB27A mutation. A diagnosis of GS2 was established. The patient received bone marrow transplantation (BMT) from an unrelated donor, and after 72 months he did not show relapse of HLH. The long, uneventful follow-up supports the use of BMT from an unrelated donor if transplantation from a relative is not possible.

Unusual patterns of presentation of frontal fibrosing alopecia: A clinical and trichoscopic analysis of 98 patients

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Ehlers-Danlos syndrome: Case report and an electron microscopy study

Ehlers–Danlos syndrome (EDS) type III is a inherited connective tissue disorders characterized by extensibility of the skin, hypermobility of the joints, chronic pain, tissue fragility, easy bruising, and delayed wound healing with result of atrophic scars. The patients report commonly a history of recurrent dislocations of the shoulders and knees after low-impact trauma, chronic joint pain, and early osteoarthritis, which lead to diagnosis. The pathogenesis of this condition is unknown, and the diagnosis is generally made in adult age, based only on clinical criteria. In this report, we describe a case of a 50-year-old woman with a 30-year history of recurrent dislocations and atrophic scars. We performed diagnosis of EDS type III after a complete clinical and instrumental evaluation, comprising of histological and electron microscopic studies, that highlighted collagen abnormalities.